Long QT syndrome

Cellular basis and arrhythmia mechanism in LQT2

Craig T. January, Qiuming Gong, Zhengfeng Zhou

Research output: Contribution to journalArticle

93 Citations (Scopus)

Abstract

HERG Channel Dysfunction in LQT2. LQT2 is one form of the congenital long QT syndrome. It results from mutations in the human ether-a-go-go-related gene (HERG), and more than 80 mutations, usually causing single amino acid substitutions in the HERG protein, are known. HERG encodes the ion channel pore-forming subunit protein for the rapidly activating delayed rectifier K+ channel (IKr) in the heart. This review summarizes current findings about mutations causing LQT2, the mechanisms by which mutations may cause the clinical phenotype of a reduction in IKr and a prolonged QT interval, and how this may be involved in the generation of ventricular arrhythmias.

Original languageEnglish (US)
Pages (from-to)1413-1418
Number of pages6
JournalJournal of Cardiovascular Electrophysiology
Volume11
Issue number12
StatePublished - 2000
Externally publishedYes

Fingerprint

Long QT Syndrome
Cardiac Arrhythmias
Ether
Mutation
Genes
Protein Subunits
Amino Acid Substitution
Ion Channels
Phenotype
Proteins

Keywords

  • Arrhythmia
  • Cardiac action potentials
  • HERG
  • Long QT syndrome
  • Potassium channels
  • Sudden death

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology

Cite this

Long QT syndrome : Cellular basis and arrhythmia mechanism in LQT2. / January, Craig T.; Gong, Qiuming; Zhou, Zhengfeng.

In: Journal of Cardiovascular Electrophysiology, Vol. 11, No. 12, 2000, p. 1413-1418.

Research output: Contribution to journalArticle

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